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Medline ® Abstract for Reference 59

of 'Molecular pathogenesis of exocrine pancreatic cancer'

59
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Very high risk of cancer in familial Peutz-Jeghers syndrome.
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Giardiello FM, Brensinger JD, Tersmette AC, Goodman SN, Petersen GM, Booker SV, Cruz-Correa M, Offerhaus JA
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Gastroenterology. 2000;119(6):1447.
 
BACKGROUND& AIMS: The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary.
METHODS: We performed an individual patient meta-analysis to determine the relative risk (RR) of cancer in patients with PJS compared with the general population based on 210 individuals described in 6 publications.
RESULTS: For patients with PJS, the RR for all cancers was 15.2 (95% confidence limits [CL], 2, 19). A statistically significant increase of RR was noted for esophagus (57; CL, 2.5, 557), stomach (213; CL, 96, 368), small intestine (520; CL, 220, 1306), colon (84; CL, 47, 137), pancreas (132; CL, 44, 261), lung (17.0; CL, 5.4, 39), breast (15.2; CL, 7.6, 27), uterus (16.0; CL, 1.9, 56), ovary (27; CL, 7.3, 68), but not testicular or cervical malignancies. Cumulative risk for all cancer was 93% from age 15 to 64 years old.
CONCLUSIONS: Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers.
AD
Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
PMID